Article
An adverse property of a familial ALS-linked SOD1 mutation causes motor neuron disease characterized by vacuolar degeneration of mitochondria.
Neuron - 1 Jun 1995
Wong P C, Pardo C A, Borchelt D R, Lee M K, Copeland N G, Jenkins N A, Sisodia S S, Cleveland D W, Price D L
Abstract excerpt
Mutations in Cu/Zn superoxide dismutase (SOD1) cause a subset of cases of familial amyotrophic lateral sclerosis. Four lines of mice accumulating one of these mutant proteins (G37R) develop severe, progressive motor neuron disease. At lower levels of mutant accumulation, pathology is restricted t...
Topics
- Amyotrophic Lateral Sclerosis
- Animals
- Axons
- Dendrites
- Humans
- Immunoenzyme Techniques
- Mice
- Mice, Inbred C3H
- Mice, Inbred C57BL
- Mice, Transgenic
- Microscopy, Electron
- Mitochondria
- Motor Neuron Disease
- Motor Neurons
- Mutation